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Gene

LMNB1

lamin B1

Summary

Cytogenetic location
5q23.2
Coordinates
Chromosome 5: 126,777,136–126,837,020 (GRCh38)
Exons
11 exons across 59.9 kb
Protein
586 amino acids
Inheritance
Autosomal dominant
MANE Select transcript
ENST00000261366.10NM_005573.4
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • Adult-Onset Leukodystrophies (CSF1R, LMNB1, Nasu-Hakola)

    • LMNB1-related adult-onset autosomal dominant leukodystrophyAutosomal dominant

      Extra copies of LMNB1, or deletions near it, cause a slowly progressive leukodystrophy beginning in mid adulthood, often starting with bladder, bowel and blood pressure control problems followed by ataxia and spasticity.

      • 2 GenCC submitters rate it definitive or strong

Broader groupings

  • Other Inherited Leukodystrophies

    • Leukodystrophy (other and unspecified types)

      One of 80 genes linked to it

      Inherited conditions that damage or prevent the normal formation of myelin, the white matter of the brain and spinal cord. Different types follow autosomal recessive, autosomal dominant or X-linked inheritance depending on the gene involved.

      • 2 GenCC submitters rate it definitive or strong
      What knowing changes

      Knowing the specific genetic cause can end a diagnostic search, guide supportive care and surveillance, show whether a specific treatment exists, and allow testing of relatives.

Inheritance

Autosomal dominant
One changed copy, from either parent, is enough. A parent who has one passes it on to each child with a one-in-two chance.

How LMNB1 is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome 5, with a bracket where LMNB1 sits. LMNB1 and 7 genes near it on chromosome 5, from 126,307,078 to 127,307,077.Chromosome 55q23.2p15.33p15.32p15.31p15.2p15.1p14.3p14.2p14.1p13.3p13.2p13.1p12p11q11.1q11.2q12.1q12.2q12.3q13.1q13.2q13.3q14.1q14.2q14.3q15q21.1q21.2q21.3q22.1q22.2q22.3q23.1q23.2q23.3q31.1q31.2q31.3q32q33.1q33.2q33.3q34q35.1q35.2q35.3126.4 Mb126.6 Mb126.8 Mb127 Mb127.2 MbGRAMD2B: 126,360,132–126,498,600, forward strandGRAMD2BALDH7A1: 126,531,200–126,595,362, reverse strandALDH7A1PHAX: 126,600,925–126,627,252, forward strandPHAXSPMIP10: 126,631,705–126,636,284, forward strandSPMIP10LMNB1: 126,777,136–126,837,020, forward strandLMNB1MARCHF3: 126,867,714–127,031,652, reverse strandMARCHF3C5orf63: 127,044,770–127,073,577, reverse strandC5orf63MEGF10: 127,290,796–127,465,737, forward strandMEGF10Diagram of LMNB1: 11 exons on the forward strand of chromosome 5Exon 1: 3 known disease-causing sitesExon 1: 126,777,136–126,777,867, 732 basesExon 2: 126,804,776–126,804,932, 157 basesExon 3: 126,805,571–126,805,696, 126 basesExon 4: 126,810,180–126,810,350, 171 basesExon 5: 126,811,773–126,811,898, 126 basesExon 6: 126,818,922–126,819,142, 221 basesExon 7: 126,820,910–126,821,135, 226 basesExon 8: 126,822,781–126,822,885, 105 basesExon 9: 126,825,988–126,826,107, 120 basesExon 10: 126,832,694–126,832,801, 108 basesExon 11: 126,836,223–126,837,020, 798 bases1246810115′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 2.9 kb of the gene's 59.9 kb; 1,761 of those bases code for the protein.

How a genome test reads LMNB1

4

positions in LMNB1 where ClinVar classes a change as pathogenic or likely pathogenic

None of them falls in a region short reads find hard.

A whole genome covers all 59.9 kb of LMNB1, introns included, not only the 2.9 kb of exons.

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). LMNB1 gene: lamin B1. Retrieved 17 October 2026, from https://dantelabs.com/genes/lmnb1

Vancouver

Dante Labs. LMNB1 gene: lamin B1 [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/lmnb1

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